Bhatti M A, Ferrante J W, Gielchinsky I, Norman J C
Ann Thorac Surg. 1985 Oct;40(4):398-401. doi: 10.1016/s0003-4975(10)60078-1.
Congenital lymphangiomatosis of lung and bone, with or without chylothorax, is a rare but often fatal systemic lymphatic malformation. In those who survive infancy and early childhood, parietal pleurectomy with excision of lymphatic lakes and ligation of the thoracic duct can be successful. Two patients with lymphangiomatosis are described, 1 with chylothorax and chylopericardium with generalized skeletal lesions and the other with pleuropulmonary lesions and chylothorax. Both were successfully treated with parietal pleurectomy, excision of lymphatic lakes, and ligation of lymphatics, including the thoracic duct. To our knowledge, the triad of generalized skeletal lymphangiomatosis, chylopericardium, and chylothorax has not been previously reported.
伴有或不伴有乳糜胸的肺和骨先天性淋巴管瘤病是一种罕见但往往致命的系统性淋巴管畸形。对于那些在婴儿期和幼儿期存活下来的患者，行胸膜壁层切除术、切除淋巴湖并结扎胸导管可能会成功。本文描述了两名淋巴管瘤病患者，1例伴有乳糜胸和乳糜心包且有全身性骨骼病变，另1例有胸膜肺病变和乳糜胸。两人均通过胸膜壁层切除术、切除淋巴湖以及结扎包括胸导管在内的淋巴管而成功治愈。据我们所知，全身性骨骼淋巴管瘤病、乳糜心包和乳糜胸这三联征此前尚未见报道。
